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Asymptomatic uveitis in young people with inflammatory bowel disease
Asymptomatic Uveitis in Young People with Inflammatory Bowel Disease Paul J. Rychwalski, M D , a Oscar A. Cruz, M D , a,b Gerardo Alanis-Lambreton, M D , a T h o m a s M. Foy, M D , b and Robert E. Kane, M D b
Purpose:To ascertain the prevalence of uveitis in a population of pediatric patients with inflammatory bowel disease without ocular symptoms. Methods: We prospectively evaluated all young people who came to the pediatric gastroenterology clinic with endoscopically proven inflammatory bowel disease between March 1994 and June 1995. All the patients were examined for evidence of ocular manifestations of inflammatory bowel disease. The examination consisted of slit-lamp examination, tonometry, and indirect ophthalmoscopy. None of the patients had visual or ocular symptoms. Eighteen patients had Crohn's disease and 14 had ulcerative colitis. Results:Of the 32 patients evaluated, four (12.5%) had evidence of asymptomatic ocular inflammation, defined as anterior chamber cell and flare. All patients with ocular inflammation were male. Three of these four male patients had Crohn's disease; the other had ulcerative colitis. Five patients had posterior subcapsular cataract, one had esotropia and amblyopia, and one had unilateral high myopia. Conclusions:The prevalence of asymptomatic uveitis in our population of young people with inflammatory bowel disease was 12.5%. These findings suggest the need for a screening ophthalmologic examination to rule out occult eye disease in young people with inflammatory bowel disease. (J AAPOS 1997;1:111-4)
n 1925, Crohn ~ described symptomatic iritis in two patients with ulcerative colitis. Numerous publications in both the ophthalmologic and the gastroenterologic literature have described an association between inflammatory bowel disease and symptomatic anterior uveitis. Various authors have placed the prevalence of symptomatic uveitis among young people with inflammatory bowel disease at 1% to 4%2-1°; it is not known, however, how many cases of inflammatory bowel disease are complicated by asymptomatic anterior uveitis. Two previous studies reported prevalences ranging between 6% and 23 %.H, 12 The long-term ocular effects of asymptomatic uveitis are unknown. It is also not known whether asymptomatic anterior uveitis associated with inflammatory bowel disease necessarily leads to symptomatic ocular inflammation, with its potential for long-term ocular damage. The purpose of this study was determine the prevalence of asymptomatic anterior uveitis in a pediatric population with inflammatory bowel disease.
i
PATIENTS AND METHODS Thirty-two consecutive patients with clinical, radiologic, and histologic evidence of inflammatory bowel disease underwent ophthalmologic examination in the Pediatric Ophthalmology Clinic of Cardinal Glennon Children's Hospital between March 1994 and June 1995. No patient had symptoms of uveitis, such as redness, pain, photophobia, or visual disturbance. Disease activity was determined by one of two gastroenterologists and included the presence of more than three stools per day, significant abdominal distress, hematochezia, and the inability to participate in normal daily activities such as school and play. The eye examination consisted of age-appropriate visual acuity testing, slit-lamp examination, tonometry, and dilated fundoseopy. The diagnosis of anterior uveitis was defined by the presence of anterior chamber cell and flare, as determined by slit-lamp examination. The degree of anterior chamber cell and flare was graded on the 0 to 4 system suggested by Kimura et a1.13Patients had a diagnosis of anterior chamber inflammation if they had grade 1 or greater cell and flare. RESULTS
From the Department of Ophthalmology, St. Louis University Eye Institute, St. Louis University School of Medicine, ~ and the Department of Pediatrics, Cardinal Glennon Children's Hospital, St. Louis. ~ Reprint requests: Oscar A. Cruz, MD, Anheuser-Busch Eye Institute, 17Y J" S. Grand Blvd., St. Louis, MO 63104. Copyright © 1997 by the American Associationfor Pediatric Ophthalmology and Strabi~'mus. !091-8Y31/97 SY.O0 + 0 75/1/80302
~ournal ofAAPOS
The mean age of our patients was 10 years (range 1 to 19 years). Twenty-six patients were white and six were black. Twenty-three of the patients were male and nine were female. Of the 32 young people, 18 had Crohn's disease and 14 had ulcerative colitis. Twenty-two patients (69%) had J~une 1997 I 11
112
Journal ofAAPOS Volume 1 Number 2June 1997
Rycbwalski etal.
TaBle 1. Characteristicsof patients with inflammatory bowel disease and asymptomatic uveitis Pt.
Age (yr)
Sex
Ethnicity
1 16 M B 2 16 M W 3 15 M W 4 8 M W IBE Inflammatorybowel disease;M, male;B, black; W,white.
IBF
Active disease
Steroid therapy
Ocular finding
Crohn's disease Crohn's disease Crohn's disease Ulcerative colitis
Yes No Yes Yes
Yes No Yes Yes
Cell and flare Cell and flare Cell and flare Cell and flare
Active disease
Steroid therapy
Ocular finding
Yes Yes Yes Yes Yes Yes Yes
PSC PSC PSC PSC PSC Esotropia/amblyopia Unilateral high myopia
Table 2. Ophthalmologicfindings of patients with inflammatory bowel diseasewithout uveitis Pt.
Age (yr)
Sex
Ethnicity
IBF
5 18 M W Crohn's disease Yes 6 17 M W Crohn's disease Yes 7 14 M W Ulcerative colitis Yes 8 tl F B Ulcerative colitis Yes 9 10 M B Crohn's disease Yes 10 17 M W Crohn's disease Yes 11 16 M W Ulcerative colitis Yes IBF,,Inflammatorybowel disease;M, Male; W,white; PSC, posteriorsubcapsularcataract; F,,female;B, black.
active inflammatory bowel disease. Four of 32 (12.5%) patients with inflammatory bowel disease had evidence by slit-lamp examination of anterior uveitis (Table 1). These patients had at least grade 1 cell and flare; no patient had greater than grade 3 cell and flare. No patient had ocular signs or symptoms of redness, pain, photophobia, or blurred vision, nor did any have other ocular signs of inflammation, such as posterior synechiae, keratic precipitates, vitreous cell, cataract, or retinal findings. Of the four patients with uveitis, three had Crohn's disease and the other had ulcerative colitis. Three of the young people with uveitis were white and the other was black. All four were male. Among the patients with asymptomatic uveitis, the average age at diagnosis of inflammatory bowel disease was 12 years and that at diagnosis of uveitis was 13.5 years. The average time between the diagnosis of inflammatory bowel disease and the finding of asymptomatic uveitis on screening ophthalmologic examination was 2 years. None of the patients had a diagnosis ofuveitis before their diagnosis of inflammatory bowel disease. Three of the four young people with uveitis had active inflammatorybowel disease and were being treated with oral prednisone. The other male patient had his oral steroid treatment discontinued 2 years before this study and was being treated with mesalamine. The dose range ofprednisone taken by the patients with asymptomatic uveitis and inflammatory bowel disease was 10 to 20 rag/day (average dose 15 nag/day). Additionally, other oral antiinflammatory and immunosuppressant medications were used in disease management, including olsalazine, mesalamine, sulfasalazine, and azathioprine. An additional seven young people with active inflammatory bowel disease had abnormal results of ophthalmologic examination (Table 2). Five young people were found to
have posterior subcapsular cataract. All of these patients had a history of prednisone therapy, with an average daily dose of 17.4 mg and a mean of 2 years, 7 months of treatment. One patient had esotropia with mild strabismic amblyopia and another had previously undiagnosed high myopia. DISCUSSION Inflammatory bowel disease describes a group Of chronic, idiopathic inflammatory disorders Of the alimentary tract. Crohn's disease and ulcerative colitis account for most inflammatory bowel disease in young people. The inflammatory process may involve the oral mucosa, esophagus, stomach, and small and large bowels. These diseases are more common in whites than in blacks, Asians, and Hispanics. Males and females are affected equally. The peak occurrence is between the ages of 15 and 35 years. 14 Two recent studies is, 16 have shown that 25% of all new cases of inflammatory bowel disease occur in those younger than 20 years old. Most of these cases are diagnosed in the second decade of the patient's life. The incidence in the pediatric population (up to 18 years) is 1.7 to 3.5 cases/ 100,000 persons/year. The cause of inflammatory bowel disease is unknown; however, genetic, infectious, immunologic, and psychologic factors may play a role in the pathogenesis. Two theories on the cause of inflammatorybowel disease prevail. The first states that the person with inflammatory bowel disease has an appropriate immune response to an unknown infection, antigen, or toxin that leads to gastrointestinal inflammation. The second theory proposes that the disease is the result of an abnormal tissue response to normal luminal factors (that is, the defect is primarily in the host immune system)?4
Journal ofAAPOS Volume 1 Number 2 June I997 In ulcerative colitis, inflammation affects only the colonic mucosa. The inflammation is uniform, is continuous (with no intervening areas of normal mucosa), and does not involve other layers of the bowel wall. In contrast, Crohn's disease is characterized by inflammation that extends through the entire wall of the affected digestive organ. The naesentery and adjacent lymph nodes may also be involved. Crohn's disease is often discontinuous; involved segments may be separated by"skip areas" of normal tissue. Granulomas may be present in Crohn's disease, but not in ulcerative colitis. Many extraintestinal manifestations of inflammatory bowel disease exist. Joint inflammation is seen in 2 5 % of patients, and skin lesions may develop in 15% of those affected. Additionally, liver function abnormalities may be seen. Ocular manifestations ofinflarnmatory bowel disease are seen in approximately 5% to 10% of adult cases, and both the anterior and posterior segments may be affected. The pathogenesis of extraintestinal manifestations of inflammatory bowel disease is purported by some to be a combination of allergic and immune responses and antibody-antigen complex deposition in extraintestinal tissues. Others believe that the diseased gastrointestinal tract may allow toxins and antigens access to the bloodstream, inviting an immunologic response. The reported anterior ocular manifestations include conjunctivitis, anterior uveitis, episcleritis, and sterile marginal corneal infiltrates. Many posterior segment manifestations have been described.S These include central serous retinochoroidopathy, panuveitis, choroiditis, ischemic optic neuropathy, retinal vasculitis, neuroretinitis, and intermediate uveitis. The prevalence of symptomatic uveitis among young people has been reported to be between 1% and 4%, but great variation exists in the pediatric literature and there are only rare mentions in ophthalmologic journals. Three articles in the pediatric literature 3,7,9. reported on studies of all extraintestinal manifestations of inflammatory bowel disease and found that only one patient in 300 had uveitis. Two previous studies, 11,12again in the pediatric literature, looked at the prevalence of asymptomatic uveitis among young people with inflammatory bowel disease. Hofley et alJ 2 found asymptomatic uveitis in six of 97 patients (6%). None of their patients showed signs of cataract formation. Also, no increased incidence of uveitis with respect to activity of bowel disease, age, or sex was noted. In 1979, Daum et al. 11 reported the prevalence of asymptomatic uveitis in their pediatric study group as 2 3 % (6/26). In both studies, all young people with asymptomatic uveitis had Crohn's disease and none had a diagnosis of ulcerative colitis. The prevalence ofasymptomatic uveitis in our screening of 32 young people with inflammatory bowel disease was 12.5% overall; 17% among patients with Crohn's disease and 7% among those with ulcerative colitis. Another five
Rychwalski et al.
113
young people had evidence of cataract. This was presumably a result of medical treatment, because all the patients with cataract had a history of oral prednisone therapy. It is unclear whether asymptomatic uveitis is a precursor to a more potentially destructive and symptomatic disease. The natural history of this entity is not known but deserves attention. It is possible that anterior uveitis is occult and recurrent in its initial stages but may then progress to a more severe, symptomatic inflammatory process. Alternatively, the uveitis may be asymptomatic and transient, causing neither symptoms nor sequelae during its entire natural course. The results of severe uveitis, however, can be devastating. Recurrent, severe uveitis leading to lightperception vision has been reported. 1° Additionally, complications resulting from the treatment of the inflammatory conditions have led to poor visual outcomes? Iris atrophy and pigment deposition can occur. Cataracts, synechiae formation, and glaucoma can result in visual loss. For this reason, asymptomatic uveitis should be screened for, and on its discovery should be watched closely. Seven of our 32 patients (22%) had other ophthalmologic abnormalities discovered during the screening examination. Five of the seven had posterior subcapsular cataract, presumably resulting from oral steroid treatment. Because cataract is a known adverse effect of oral steroid therapy, pediatric patients with a history of such therapy would also benefit from yearly ophthalmologic examination. Such examination is especially important in young children (up to 9 years), in whom failure to detect a cataract could lead to deprivational amblyopia. Our data suggest the need for routine ophthalmologic screening in young people with inflammatory bowel disease. The long-term significance for ocular health of asymptomatic uveitis in young people with inflammatory bowel disease is not known. Additional follow-up data are needed, and more patients must be examined. Significant visual morbidity may be prevented by early detection and treatment of the uveitis seen in young people with inflammatory bowel disease. Screening will also provide an opportunity to intervene early in the disease course in case it should eventually progress to a more severe and vision-threatening stage.
References
1. Crohn BB. Ocularlesionscomplicatingulcerativecolitis.AmJ Med Sci 1925;169:260-7. 2. Billson FA, deDombal FT, Watldnson G, Goligher JC. Ocular complications of ulcerative colitis. Gut 1967;8:102-6. 3. DanziJT. Extraintestinal manifestationsof idiopathicinflammatory boweldisease.&rchIntern Med 1988;148:297-301. 4. EllisPP, GentxyJH.Ocularcomplicationsofulcerativecolitis.AmJ Ophthalmol 1964;58:779-85. 5. Ernst BB, LowderCY,MeislerSM, GutmanFA. Posteriorsegment manifestations of inflammatory bowel disease. Ophthalmology 1991;98:1272-80. 6. Hopkins DJ, Horan E, Burton IL, Champ SE, DeDombal FT, Goligher JC. Ocular disorders in a series of 332 patients with Crohn's disease.BrJ Ophthalmol 1974;58:732-7.
114
Journal ofAAPOS Volume 1 Number 2June 1997
Rychwalski et al.
7. Hyams JS. Extraintestinal manifestations of inflammatory bowel disease in children. J Pediatr Gastroenterol Nutr 1994;1:7-21. 8. Knox DL, Schachat AP, Mustonen E. Primary, secondary and coincidental ocular complications of Crohn's disease. Ophthalmology 1984;91:163-73. 9. Pettei MJ, Davidson M. Extra-gastrointestinal manifestations of inflammatory bowel disease [editorial]. J Pediatr Gastroenterol Nutr 1985;4:689-91. 10. Salmon JF, Wright JP, Murray ADN. Ocular inflammation in Crohn's disease. Ophthalmology 1991;98:480-4. 11. Daum F, Gould FIB, Gold D, Dinari G, Friedman AH, Zncker P, et al. Asymptomatic transient uveitis in children with inflammatory bowel disease. AmJ Dis Child 1979;133:170-1. 12. Hofley P, Roarty J, McGinnity G, Griffiths AM, Marcon M.
13. 14.
15.
16.
Asymptomatic uveitis in children with chronic inflammatory bowel disease. J Pediatr Gastroenterol Nutr 1993;17:397-400. Kimura SJ, Thygeson P, Hogan MJ. Signs and symptoms ofuveitis. I. Anterior uveitis. Am J Ophthalmol 1959;47:155-70. Leichtner AM, Jackson WD, Grand RJ. Crohn's disease. In: Walker WA, Durie PR, Hamilton JR, Walker-Smith JA, Watldns JB, editors. Pediatric gastrointestinal disease, vol 1.2nd ed. St Louis: Mosby; 1996. Chap. 27. Hildebrand H, Fredrikzon B, Holmquist L, Kristiansson B, Lindquist B. Chronic inflammatory bowel disease in children and adolescents in Sweden. J Pediatr Gastroenterol Nntr 1991;13:293 -7. Calkins BM, Lilienfield AM, Garland CF, Mendeloff AI. Trends in incidence rates of ulcerative colitis and Crohn's disease. Dig Dis Sci 1984;29:913-20.
Purpose:To ascertain the prevalence of uveitis in a population of pediatric patients with inflammatory bowel disease without ocular symptoms. Methods: We prospectively evaluated all young people who came to the pediatric gastroenterology clinic with endoscopically proven inflammatory bowel disease between March 1994 and June 1995. All the patients were examined for evidence of ocular manifestations of inflammatory bowel disease. The examination consisted of slit-lamp examination, tonometry, and indirect ophthalmoscopy. None of the patients had visual or ocular symptoms. Eighteen patients had Crohn's disease and 14 had ulcerative colitis. Results:Of the 32 patients evaluated, four (12.5%) had evidence of asymptomatic ocular inflammation, defined as anterior chamber cell and flare. All patients with ocular inflammation were male. Three of these four male patients had Crohn's disease; the other had ulcerative colitis. Five patients had posterior subcapsular cataract, one had esotropia and amblyopia, and one had unilateral high myopia. Conclusions:The prevalence of asymptomatic uveitis in our population of young people with inflammatory bowel disease was 12.5%. These findings suggest the need for a screening ophthalmologic examination to rule out occult eye disease in young people with inflammatory bowel disease. (J AAPOS 1997;1:111-4)
n 1925, Crohn ~ described symptomatic iritis in two patients with ulcerative colitis. Numerous publications in both the ophthalmologic and the gastroenterologic literature have described an association between inflammatory bowel disease and symptomatic anterior uveitis. Various authors have placed the prevalence of symptomatic uveitis among young people with inflammatory bowel disease at 1% to 4%2-1°; it is not known, however, how many cases of inflammatory bowel disease are complicated by asymptomatic anterior uveitis. Two previous studies reported prevalences ranging between 6% and 23 %.H, 12 The long-term ocular effects of asymptomatic uveitis are unknown. It is also not known whether asymptomatic anterior uveitis associated with inflammatory bowel disease necessarily leads to symptomatic ocular inflammation, with its potential for long-term ocular damage. The purpose of this study was determine the prevalence of asymptomatic anterior uveitis in a pediatric population with inflammatory bowel disease.
i
PATIENTS AND METHODS Thirty-two consecutive patients with clinical, radiologic, and histologic evidence of inflammatory bowel disease underwent ophthalmologic examination in the Pediatric Ophthalmology Clinic of Cardinal Glennon Children's Hospital between March 1994 and June 1995. No patient had symptoms of uveitis, such as redness, pain, photophobia, or visual disturbance. Disease activity was determined by one of two gastroenterologists and included the presence of more than three stools per day, significant abdominal distress, hematochezia, and the inability to participate in normal daily activities such as school and play. The eye examination consisted of age-appropriate visual acuity testing, slit-lamp examination, tonometry, and dilated fundoseopy. The diagnosis of anterior uveitis was defined by the presence of anterior chamber cell and flare, as determined by slit-lamp examination. The degree of anterior chamber cell and flare was graded on the 0 to 4 system suggested by Kimura et a1.13Patients had a diagnosis of anterior chamber inflammation if they had grade 1 or greater cell and flare. RESULTS
From the Department of Ophthalmology, St. Louis University Eye Institute, St. Louis University School of Medicine, ~ and the Department of Pediatrics, Cardinal Glennon Children's Hospital, St. Louis. ~ Reprint requests: Oscar A. Cruz, MD, Anheuser-Busch Eye Institute, 17Y J" S. Grand Blvd., St. Louis, MO 63104. Copyright © 1997 by the American Associationfor Pediatric Ophthalmology and Strabi~'mus. !091-8Y31/97 SY.O0 + 0 75/1/80302
~ournal ofAAPOS
The mean age of our patients was 10 years (range 1 to 19 years). Twenty-six patients were white and six were black. Twenty-three of the patients were male and nine were female. Of the 32 young people, 18 had Crohn's disease and 14 had ulcerative colitis. Twenty-two patients (69%) had J~une 1997 I 11
112
Journal ofAAPOS Volume 1 Number 2June 1997
Rycbwalski etal.
TaBle 1. Characteristicsof patients with inflammatory bowel disease and asymptomatic uveitis Pt.
Age (yr)
Sex
Ethnicity
1 16 M B 2 16 M W 3 15 M W 4 8 M W IBE Inflammatorybowel disease;M, male;B, black; W,white.
IBF
Active disease
Steroid therapy
Ocular finding
Crohn's disease Crohn's disease Crohn's disease Ulcerative colitis
Yes No Yes Yes
Yes No Yes Yes
Cell and flare Cell and flare Cell and flare Cell and flare
Active disease
Steroid therapy
Ocular finding
Yes Yes Yes Yes Yes Yes Yes
PSC PSC PSC PSC PSC Esotropia/amblyopia Unilateral high myopia
Table 2. Ophthalmologicfindings of patients with inflammatory bowel diseasewithout uveitis Pt.
Age (yr)
Sex
Ethnicity
IBF
5 18 M W Crohn's disease Yes 6 17 M W Crohn's disease Yes 7 14 M W Ulcerative colitis Yes 8 tl F B Ulcerative colitis Yes 9 10 M B Crohn's disease Yes 10 17 M W Crohn's disease Yes 11 16 M W Ulcerative colitis Yes IBF,,Inflammatorybowel disease;M, Male; W,white; PSC, posteriorsubcapsularcataract; F,,female;B, black.
active inflammatory bowel disease. Four of 32 (12.5%) patients with inflammatory bowel disease had evidence by slit-lamp examination of anterior uveitis (Table 1). These patients had at least grade 1 cell and flare; no patient had greater than grade 3 cell and flare. No patient had ocular signs or symptoms of redness, pain, photophobia, or blurred vision, nor did any have other ocular signs of inflammation, such as posterior synechiae, keratic precipitates, vitreous cell, cataract, or retinal findings. Of the four patients with uveitis, three had Crohn's disease and the other had ulcerative colitis. Three of the young people with uveitis were white and the other was black. All four were male. Among the patients with asymptomatic uveitis, the average age at diagnosis of inflammatory bowel disease was 12 years and that at diagnosis of uveitis was 13.5 years. The average time between the diagnosis of inflammatory bowel disease and the finding of asymptomatic uveitis on screening ophthalmologic examination was 2 years. None of the patients had a diagnosis ofuveitis before their diagnosis of inflammatory bowel disease. Three of the four young people with uveitis had active inflammatorybowel disease and were being treated with oral prednisone. The other male patient had his oral steroid treatment discontinued 2 years before this study and was being treated with mesalamine. The dose range ofprednisone taken by the patients with asymptomatic uveitis and inflammatory bowel disease was 10 to 20 rag/day (average dose 15 nag/day). Additionally, other oral antiinflammatory and immunosuppressant medications were used in disease management, including olsalazine, mesalamine, sulfasalazine, and azathioprine. An additional seven young people with active inflammatory bowel disease had abnormal results of ophthalmologic examination (Table 2). Five young people were found to
have posterior subcapsular cataract. All of these patients had a history of prednisone therapy, with an average daily dose of 17.4 mg and a mean of 2 years, 7 months of treatment. One patient had esotropia with mild strabismic amblyopia and another had previously undiagnosed high myopia. DISCUSSION Inflammatory bowel disease describes a group Of chronic, idiopathic inflammatory disorders Of the alimentary tract. Crohn's disease and ulcerative colitis account for most inflammatory bowel disease in young people. The inflammatory process may involve the oral mucosa, esophagus, stomach, and small and large bowels. These diseases are more common in whites than in blacks, Asians, and Hispanics. Males and females are affected equally. The peak occurrence is between the ages of 15 and 35 years. 14 Two recent studies is, 16 have shown that 25% of all new cases of inflammatory bowel disease occur in those younger than 20 years old. Most of these cases are diagnosed in the second decade of the patient's life. The incidence in the pediatric population (up to 18 years) is 1.7 to 3.5 cases/ 100,000 persons/year. The cause of inflammatory bowel disease is unknown; however, genetic, infectious, immunologic, and psychologic factors may play a role in the pathogenesis. Two theories on the cause of inflammatorybowel disease prevail. The first states that the person with inflammatory bowel disease has an appropriate immune response to an unknown infection, antigen, or toxin that leads to gastrointestinal inflammation. The second theory proposes that the disease is the result of an abnormal tissue response to normal luminal factors (that is, the defect is primarily in the host immune system)?4
Journal ofAAPOS Volume 1 Number 2 June I997 In ulcerative colitis, inflammation affects only the colonic mucosa. The inflammation is uniform, is continuous (with no intervening areas of normal mucosa), and does not involve other layers of the bowel wall. In contrast, Crohn's disease is characterized by inflammation that extends through the entire wall of the affected digestive organ. The naesentery and adjacent lymph nodes may also be involved. Crohn's disease is often discontinuous; involved segments may be separated by"skip areas" of normal tissue. Granulomas may be present in Crohn's disease, but not in ulcerative colitis. Many extraintestinal manifestations of inflammatory bowel disease exist. Joint inflammation is seen in 2 5 % of patients, and skin lesions may develop in 15% of those affected. Additionally, liver function abnormalities may be seen. Ocular manifestations ofinflarnmatory bowel disease are seen in approximately 5% to 10% of adult cases, and both the anterior and posterior segments may be affected. The pathogenesis of extraintestinal manifestations of inflammatory bowel disease is purported by some to be a combination of allergic and immune responses and antibody-antigen complex deposition in extraintestinal tissues. Others believe that the diseased gastrointestinal tract may allow toxins and antigens access to the bloodstream, inviting an immunologic response. The reported anterior ocular manifestations include conjunctivitis, anterior uveitis, episcleritis, and sterile marginal corneal infiltrates. Many posterior segment manifestations have been described.S These include central serous retinochoroidopathy, panuveitis, choroiditis, ischemic optic neuropathy, retinal vasculitis, neuroretinitis, and intermediate uveitis. The prevalence of symptomatic uveitis among young people has been reported to be between 1% and 4%, but great variation exists in the pediatric literature and there are only rare mentions in ophthalmologic journals. Three articles in the pediatric literature 3,7,9. reported on studies of all extraintestinal manifestations of inflammatory bowel disease and found that only one patient in 300 had uveitis. Two previous studies, 11,12again in the pediatric literature, looked at the prevalence of asymptomatic uveitis among young people with inflammatory bowel disease. Hofley et alJ 2 found asymptomatic uveitis in six of 97 patients (6%). None of their patients showed signs of cataract formation. Also, no increased incidence of uveitis with respect to activity of bowel disease, age, or sex was noted. In 1979, Daum et al. 11 reported the prevalence of asymptomatic uveitis in their pediatric study group as 2 3 % (6/26). In both studies, all young people with asymptomatic uveitis had Crohn's disease and none had a diagnosis of ulcerative colitis. The prevalence ofasymptomatic uveitis in our screening of 32 young people with inflammatory bowel disease was 12.5% overall; 17% among patients with Crohn's disease and 7% among those with ulcerative colitis. Another five
Rychwalski et al.
113
young people had evidence of cataract. This was presumably a result of medical treatment, because all the patients with cataract had a history of oral prednisone therapy. It is unclear whether asymptomatic uveitis is a precursor to a more potentially destructive and symptomatic disease. The natural history of this entity is not known but deserves attention. It is possible that anterior uveitis is occult and recurrent in its initial stages but may then progress to a more severe, symptomatic inflammatory process. Alternatively, the uveitis may be asymptomatic and transient, causing neither symptoms nor sequelae during its entire natural course. The results of severe uveitis, however, can be devastating. Recurrent, severe uveitis leading to lightperception vision has been reported. 1° Additionally, complications resulting from the treatment of the inflammatory conditions have led to poor visual outcomes? Iris atrophy and pigment deposition can occur. Cataracts, synechiae formation, and glaucoma can result in visual loss. For this reason, asymptomatic uveitis should be screened for, and on its discovery should be watched closely. Seven of our 32 patients (22%) had other ophthalmologic abnormalities discovered during the screening examination. Five of the seven had posterior subcapsular cataract, presumably resulting from oral steroid treatment. Because cataract is a known adverse effect of oral steroid therapy, pediatric patients with a history of such therapy would also benefit from yearly ophthalmologic examination. Such examination is especially important in young children (up to 9 years), in whom failure to detect a cataract could lead to deprivational amblyopia. Our data suggest the need for routine ophthalmologic screening in young people with inflammatory bowel disease. The long-term significance for ocular health of asymptomatic uveitis in young people with inflammatory bowel disease is not known. Additional follow-up data are needed, and more patients must be examined. Significant visual morbidity may be prevented by early detection and treatment of the uveitis seen in young people with inflammatory bowel disease. Screening will also provide an opportunity to intervene early in the disease course in case it should eventually progress to a more severe and vision-threatening stage.
References
1. Crohn BB. Ocularlesionscomplicatingulcerativecolitis.AmJ Med Sci 1925;169:260-7. 2. Billson FA, deDombal FT, Watldnson G, Goligher JC. Ocular complications of ulcerative colitis. Gut 1967;8:102-6. 3. DanziJT. Extraintestinal manifestationsof idiopathicinflammatory boweldisease.&rchIntern Med 1988;148:297-301. 4. EllisPP, GentxyJH.Ocularcomplicationsofulcerativecolitis.AmJ Ophthalmol 1964;58:779-85. 5. Ernst BB, LowderCY,MeislerSM, GutmanFA. Posteriorsegment manifestations of inflammatory bowel disease. Ophthalmology 1991;98:1272-80. 6. Hopkins DJ, Horan E, Burton IL, Champ SE, DeDombal FT, Goligher JC. Ocular disorders in a series of 332 patients with Crohn's disease.BrJ Ophthalmol 1974;58:732-7.
114
Journal ofAAPOS Volume 1 Number 2June 1997
Rychwalski et al.
7. Hyams JS. Extraintestinal manifestations of inflammatory bowel disease in children. J Pediatr Gastroenterol Nutr 1994;1:7-21. 8. Knox DL, Schachat AP, Mustonen E. Primary, secondary and coincidental ocular complications of Crohn's disease. Ophthalmology 1984;91:163-73. 9. Pettei MJ, Davidson M. Extra-gastrointestinal manifestations of inflammatory bowel disease [editorial]. J Pediatr Gastroenterol Nutr 1985;4:689-91. 10. Salmon JF, Wright JP, Murray ADN. Ocular inflammation in Crohn's disease. Ophthalmology 1991;98:480-4. 11. Daum F, Gould FIB, Gold D, Dinari G, Friedman AH, Zncker P, et al. Asymptomatic transient uveitis in children with inflammatory bowel disease. AmJ Dis Child 1979;133:170-1. 12. Hofley P, Roarty J, McGinnity G, Griffiths AM, Marcon M.
13. 14.
15.
16.
Asymptomatic uveitis in children with chronic inflammatory bowel disease. J Pediatr Gastroenterol Nutr 1993;17:397-400. Kimura SJ, Thygeson P, Hogan MJ. Signs and symptoms ofuveitis. I. Anterior uveitis. Am J Ophthalmol 1959;47:155-70. Leichtner AM, Jackson WD, Grand RJ. Crohn's disease. In: Walker WA, Durie PR, Hamilton JR, Walker-Smith JA, Watldns JB, editors. Pediatric gastrointestinal disease, vol 1.2nd ed. St Louis: Mosby; 1996. Chap. 27. Hildebrand H, Fredrikzon B, Holmquist L, Kristiansson B, Lindquist B. Chronic inflammatory bowel disease in children and adolescents in Sweden. J Pediatr Gastroenterol Nntr 1991;13:293 -7. Calkins BM, Lilienfield AM, Garland CF, Mendeloff AI. Trends in incidence rates of ulcerative colitis and Crohn's disease. Dig Dis Sci 1984;29:913-20.